Lipomyelomeningocele is the most common malformation leading to spinal cord tethering. Nonetheless, these lesion continues to pose a challenge in patient management. The purpose of this study was to identify the clinical feature, candidate for surgical repair, and outcome of lipomyelomeningocele in young children and adults. We studied the magnetic resonance images and/or lumbar myelo-CT of lipomyelomeningocele performed between January 1987 and September 1995 at our institutions and reviewed the pertinent medical records and radiologic stuies to detemine clinical feature, surgical candidate, and outcomes of these patients. Of the 14 cases(11 female and 3 males) studied, the most common factor that caused these patients to seek help was the cosmetic effect of the mass on their back, followed by urinary incontinence, and weakness of lower extremities. The patients' age ranged from 2 months to 21 years(mean, 5.0 years). All the patients underwent immediate surgical management that consisted of removal of lipoma, untethering of the cord, and complete dural sac repair. Follow up for these patients ranged from 6 to 82 months(median, 54 mo). None of the patients that underwent surgical correction before the emergence of neurological deficit had ever developed neurological deficits or uninary incontinence at the time of surgery, improved significantly. In conclusion, early detection and prophylactic surgery is imperative to prevent permanent neurological deficits and urinary dysfunction.